You are in: eMedicine Specialties > Dermatology > DISEASES OF THE VESSELS Nevus AnemicusArticle Last Updated: Feb 21, 2007AUTHOR AND EDITOR INFORMATIONSection 1 of 10
  Author: Theresa G Knoepp, MD, Consulting Staff, Anderson Dermatology and Skin Surgery Center, LLC Coauthor(s): Loretta Davis, MD, Professor, Department of Internal Medicine, Division of Dermatology, Medical College of Georgia Editors: Carrie L Kovarik, MD, Assistant Professor, Department of Dermatology and Dermatopathology, University of Pennsylvania School of Medicine; David F Butler, MD, Professor of Dermatology, Texas A&M University College of Medicine; Director, Division of Dermatology, Scott and White Clinic; Director Dermatology Residency Training Program, Scott and White Clinic; Jeffrey J Miller, MD, Associate Professor, Department of Dermatology, Penn State University, Milton S Hershey Medical Center; Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University; William D James, MD, Paul R Gross Professor of Dermatology, University of Pennsylvania School of Medicine; Vice-Chair, Program Director, Department of Dermatology, University of Pennsylvania Health System Author and Editor Disclosure Synonyms and related keywords: hypopigmentation, nevi anemicus, familial cutis tricolor, phakomatosis pigmentovascularis, benign nevi, congenital nevi INTRODUCTIONSection 2 of 10
  BackgroundNevus anemicus is a congenital localized vascular anomaly that presents clinically as a hypopigmented macule or patch (see Image 1). This disorder is due to a localized hypersensitivity to catecholamines. It is an uncommon disorder and was first described by Vorner in 1906. PathophysiologyIntralesional injection of bradykinin, acetylcholine, serotonin, nicotine, 5-hydroxytryptamine, and histamine fails to induce the anticipated vasodilatation or erythema in the affected area. However, erythema does follow an axillary sympathetic block or intradermal injection of the alpha-adrenergic blocking agent, pilocarpine. These findings suggest that nevus anemicus is best termed a pharmacologic nevus resulting from increased vascular sensitivity to catecholamines. This conclusion is further supported by autograft exchange transplantation studies that show donor site dominance. It also has been proposed that an abnormality in endothelial adhesion molecule induction (E selectin expression) may be involved, suggesting several pharmacologic anomalies are involved and further supporting the idea that nevus anemicus may best be termed a pharmacologic nevus. FrequencyUnited StatesThe prevalence of nevus anemicus is not known, but it is not rare. Mortality/MorbidityLesions of nevus anemicus usually persist unchanged throughout life. They are asymptomatic. RaceNo racial predilection has been noted in the literature. SexNevus anemicus appears more frequently in females. AgeNevus anemicus may be present at birth or appear in early childhood. CLINICALSection 3 of 10
HistoryPatients typically present with an asymptomatic pale macule or patch that has been present since birth and grows with the child. Frequently, the lesion of nevus anemicus is noted as an incidental finding on skin examination. PhysicalClinically, nevus anemicus is a circumscribed, rounded, oval or linear pale macule or patch with irregular margins that may be surrounded by satellite macules. Lesions may be single or multiple and may be located on any part of the body, but most lesions commonly are found on the upper chest. They occur more frequently in females and are usually asymptomatic. Nevus anemicus may be present at birth or appear in early childhood, although it may be easy to overlook. CausesNevus anemicus results from a congenital anomaly in which hypersensitivity is localized to catecholamines. Reports describe nevus anemicus as part of a familial cutis tricolor with a suggested paradominant inheritance. DIFFERENTIALSSection 4 of 10
Vitiligo
| ||||||||||||||
| Media file 1: Ill-defined, asymptomatic hypopigmented patch of nevus anemicus on the lateral leg. | |
 | View Full Size Image | Media type: Photo |
Article Last Updated: Feb 21, 2007
